Shulman disease

  • 文章类型: Journal Article
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  • 文章类型: Journal Article
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    目的:Shulman于1974年将嗜酸性筋膜炎(EF)描述为一种病因不明的罕见纤维化结缔组织疾病。不确定的触发因素被认为导致与成纤维细胞相互作用并表达纤维化细胞因子(包括肿瘤生长的转化因子a和b以及白细胞介素1和6)的嗜酸性粒细胞的脱颗粒。目的总结某中心医院的7例EF病例。
    方法:这是一项对中心医院EF患者的回顾性和描述性研究。在2005年1月1日至2018年4月30日期间,所有在医院单位诊断为EF的患者均被纳入研究。
    结果:共分析了7例诊断为EF的患者。诊断时的中位年龄为56岁,57%的患者是女性。所有患者外周血嗜酸性粒细胞增多和沉降率升高,只有一名患者患有高丙种球蛋白血症。所有患者均有四肢水肿和皮肤增厚,57%有体质症状,57%的人患有炎性关节炎伴关节挛缩。泼尼松龙(PDN)治疗在所有患者开始,只有两个是最初进行的PDN与甲氨蝶呤(MTX)的关联。在一名PDN三联疗法患者中,MTX,需要环孢菌素。在此发布时,只有一名患者保持活动性疾病,托珠单抗已经启动。
    结论:最近的研究表明,与单独使用PDN相比,PDN和MTX的组合反应更有利。考虑到这种疾病的稀有性,关于病因遗传学需要更多的长期研究,programming,EF复发,和新的有效疗法。
    OBJECTIVE: Eosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and express fibrogenic cytokines including the transforming factor of tumor growth a and b and interleukins 1 and 6. The purpose of this study was to summarize seven cases of EF in a central hospital.
    METHODS: This was a retrospective and descriptive study of a population with EF of a central hospital. All patients diagnosed with EF in a hospital unit were admitted to the study between January 1, 2005, and April 30, 2018.
    RESULTS: A total of seven patients diagnosed with EF were analyzed. The median age of the population at the time of diagnosis was 56 years, and 57% of the patients were women. All patients had elevated peripheral eosinophilia and sedimentation rate, and only one patient had hypergammaglobulinemia. All patients had edema and cutaneous thickening of the limbs, 57% had constitutional symptoms, and 57% had inflammatory arthritis with joint contracture. Prednisolone (PDN) therapy was initiated in all patients, and only in two was the association of PDN with methotrexate (MTX) initially performed. In one patient triple therapy of PDN, MTX, and cyclosporine was required. At the time of this publication, only one patient maintains active disease, and tocilizumab has been initiated.
    CONCLUSIONS: Recent studies show a more favorable response from the combination of PDN and MTX than from PDN alone. Considering the rarity of the disease, more long-term studies are needed regarding the etiopathogenetics, progression, recurrence of EF, and new effective therapies.
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  • 文章类型: Journal Article
    OBJECTIVE: First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF.
    RESULTS: Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response. Systemic corticosteroids remain the first-line treatment for EF; however, their often prolonged use in the treatment of EF has prompted a search for adjunctive therapies. Methotrexate has emerged as the leading corticosteroid-sparing agent for EF. Since EF was initially described over 40 years ago, important diagnostic and therapeutic progress has been made. Future efforts should be directed at the pursuit of prospective studies including clinical trials and evidence-based guidelines.
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